Synovial Sarcoma in Head and Neck:A Case ReportAdriano Santana Fonseca1 Amanda Canário Andrade Azevedo1 Fabíola Moreira Magalhães1

Nilvano Alves de Andrade1

1Santa Casa de Misericórdia da Bahia, Salvador, BA, Brazil

Int Arch Otorhinolaryngol 2014;18:87–89.

Address for correspondence Adriano Santana Fonseca, MD, Santa Casade Misericórdia da Bahia, Hospital Santa Izabel, Unidade deOtorrinolaringologia, Praça Conselheiro Almeida Couto, 500, Nazaré,Salvador, Bahia, Brazil 40050-410 (e-mail: adrianootorrino@gmail.com).

Introduction

Synovial sarcoma is a malignant tumor of pluripotent mes-enchymal cells,1–4 most commonly found in periarticularareas, with few cases occurring in the head and neck.1–5

The first synovial sarcoma was described by Jernstrom in1954.6 In this article, we present a case of synovial sarcoma inthe posterolateral wall of the oropharynx. This case reportwas authorized by the ethics committee by the number229.577.

Case Report

The patient, a 23-year-old woman, was admitted to theotorhinolaryngology clinic at our hospital with a history ofdysphagia and difficulty of breathing for 8 months, resultingin progressive worsening and emergence of snoring, muffledvoice, and local pain. She denied fever episodes, smoking, andalcohol consumption. Physical examination revealed an oro-pharyngeal tumor in the left posterolateral wall near the baseof the tongue. Rhinoscopy and otoscopy confirmed the

Keywords

► sarcoma► synovial► deglutition disorders

Abstract Introduction Synovial sarcoma is a malignant tumor of mesenchymal pluripotentcells.Objectives We present a case of synovial sarcoma in the posterolateral wall of theoropharynx.Resumed report The patient, a 23-year-old woman, was admitted with a history ofdysphagia and difficulty in breathing for 8months, resulting in progressive deteriorationand onset of snoring, muffled voice, and local pain. An oropharyngeal tumor in the leftposterolateral wall touched the base of the ipsilateral tongue. The patient underwentendoscopic pharyngectomy to remove the lesion. Pathologic examination revealedsynovial sarcoma with positive margins, and Mohs technique was proposed for margincontrol. The margins were disease-free, without the need for total laryngectomy. Thepharynx was reconstructed with a microvascular forearm flap. The patient developedpostoperative stability.Conclusion Despite its name, synovial sarcoma is rarely sourced directly from synovialmembranes. It is most commonly found in the vicinity of large joints. The location at thehead and neck, a location poor in synovial tissue, is unusual. Synovial sarcoma in thehead and neck has an aggressive nature and poor prognosis. Resection with negativemargins remains the foundation of therapy, which is not so easily achieved in the headand neck. It is important for the otorhinolaryngologist and head and neck surgeon to befamiliar with this aggressive tumor, which carries high mortality and morbidity. Theappropriate diagnosis and treatment can improve prognosis and patient survival.

receivedJune 26, 2013acceptedSeptember 10, 2013

DOI http://dx.doi.org/10.1055/s-0033-1361081.ISSN 1809-9777.

Copyright © 2014 by Thieme PublicaçõesLtda, Rio de Janeiro, Brazil

THIEME

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diagnosis, with no changes. Videolaryngoscopy (►Fig. 1)showed a rounded submucosal tumor with intact mucosa,starting at the left posterolateral wall, touching the epiglottisdown to the left, narrowing the light of the entire pharynx,preventing full view of the glottis. Cervical magnetic reso-nance imaging (►Figs. 2, 3, and 4) showed a solid cystic lesionin the submucosal left posterolateral wall of the oropharynxof relatively delimited contour, reducing the airway andrejecting the left piriform sinus. The mass measured�5.3 � 3.9 � 2.2 cm in its greatest axis. Its left lateral regionwas predominately solid, showing heterogeneous impregna-tion of paramagnetic contrast, and measured 2.1 � 2.0 cm,with the cleavage plane delimited by prevertebral muscles.Lymph nodes measured up to 1.3 cm in internal high jugularchains and submandibular (IB and IIA) on the right. Labora-tory tests were unchanged.

Endoscopic pharyngectomy was used to excise the tumor.Pathologic examination revealed a synovial sarcoma withpositive margins, and Mohs technique was proposed for

edge control. A full pharyngectomy with ipsilateral modifiedradical neck dissection was proposed, with the possibility offull laryngeal pharyngectomy depending on surgical marginsobtained intraoperatively by freezing anatomy. During sur-gery, the margins came initially committed, and after expan-sion, the margins were disease-free. It was possible to obtainfree surgical margins without the need for total laryngectomy.The pharynx was reconstructed with a microvascularizedforearm flap.We also performed the proposed neckdissection,tracheostomy, and passage of a nasogastric tube for nutrition.The neck dissection was disease-free.

Postoperatively, the patient’s condition was stable. Uponfollow-up with an oncologist, chemotherapy and radiationwere given. A gastrostomy was performed for nutrition, asoral feeding was impossible. At swallowing endoscopy, therewas saliva aspiration due to lack of mobility of the pharyngealmuscles. The patient is already in speech therapy. All mobilityof the larynx was preserved. Within�1 year postoperatively,the patient continues with locoregional control withoutrecurrence and performs pharyngoesophageal dilatations to

Fig. 1 Preoperative laryngoscopy showing the mass at thehypopharynx.

Fig. 2 Nuclear magnetic resonance, axial view.

Fig. 3 Nuclear magnetic resonance, coronal view.

Fig. 4 Nuclear magnetic resonance, sagittal view.

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treat actinic stenosis. Return to oral feeding is in progress. Thetracheostomy tube is closed and will be removed as soon asoral feeding is stabilized.

Discussion

Despite its name, synovial sarcoma rarely originates in thesynovial membranes.7 It is most commonly found in the vicinityof large joints. Occurrence in the head and neck, a location poorin synovial tissue, is uncommon.8 Synovial sarcoma comprises8 to 10% of all soft tissue sarcoma,1,3,5,7,9 and it is estimated that�3 to 10% of sarcomas occur in the head and neck.4,5,10 Thesetumors can be located in the prevertebral space from the base ofthe skull to the hypopharynx, retropharyngeal and paraphar-yngeal spaces, and anterior neck along the edges of the sterno-cleidomastoid muscle, as well as sites in the oropharynx andlarynx.8,10 Synovial sarcomas show a male predominance (3:2)and predilection for patients between 25 and 36 years ofage.4,11,12

This type of tumor usually appears as an asymptomaticmass until it acquires sufficient volume to cause compressiveeffects on neighboring structures.1 It can be a painlessmass orassociated with symptoms such as pain, earache, sore throat,and bleeding. There are two subtypes: monophasic andbiphasic. For diagnosis, it is important to include laryngosco-py and head, neck, and chest computed tomography to definethe local extent of disease and metastasis.4

Resection with negative margins remains the foundationof therapy,7,12 which is not so easy to achieve in the head andneck.4,5 Local excision is followed byhigh recurrence rates (60to 90%), usually within 2 years.4,5,9 Metastasis to regionallymph nodes of synovial sarcomas in the head and neck is notseen often, although there can be clinically enlarged lymphnodes.5 Most metastasis originate from hematogenous dis-semination, although up to 20% spread through the lym-phatics to regional lymph nodes.4,8 Cervical lymph nodedissection is not routinely performed, except in the presenceof enlarged lymph nodes.8,10Hematogenous dissemination isthe harbinger of death. Pre- and postoperative radiotherapy,with or without chemotherapy, may be responsible for theincrease in survival statistics over the last 2 to 3 decades, butstudies are still controversial and inconclusive.4,7,9

Despite relatively slow growth, long-term survival forsynovial sarcomas from anywhere, including the head andneck, is not good.5 Synovial sarcoma in the head and neck hasan aggressive nature and a reserved prognosis.3,8 Severalfactors have been evaluated for prognosis, and the mostsignificant were tumor size and deep extension at the timeof primary treatment,9 with survival varying inversely with

these two factors.5 The age at diagnosis seems to haveinvolvement with prognosis,9 although the relative youth ofpatients with synovial sarcoma of the head and neck does notseem to give a significantly better prognosis.5

Final Comments

Synovial sarcoma is a rare malignant tumor which treatmentis essentially surgical resection with high rates of relapse.Although traditional treatment for hypopharynx tumors hasbeen laryngectomy, it can be modified for sarcomas at thisarea. In these cases, most important are the border’s micro-scopic control and lack of lymphatic metastasis. Radiationtherapy with or without chemotherapy appears to be respon-sible for the increased survival in recent decades. It is impor-tant for the otolaryngologist and head and neck surgeon to befamiliar with this aggressive tumor, which carries high mor-tality and morbidity. The appropriate early diagnosis andtreatment can improve the prognosis and survival of patients.

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